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JC virus granule cell neuronopathy in the setting of chronic lymphopenia treated with recombinant interleukin-7
David N Soleimani-Meigooni1, Katherine E Schwetye2, Maria Reyes Angeles3
1Department of Neurology, Washington University School of Medicine, Campus Box 8111. 660 South Euclid Ave., St. Louis, MO, 63110, USA.
Abstract:
JC virus (JCV) is a human polyomavirus that infects the central nervous system (CNS) of immunocompromised patients. JCV granule cell neuronopathy (JCV-GCN) is caused by infection of cerebellar granule cells, causing ataxia. A 77-year-old man with iatrogenic lymphopenia presented with severe ataxia and was diagnosed with JCV-GCN. His ataxia and cerebrospinal fluid (CSF) improved with intravenous immunoglobulin, high-dose intravenous methylprednisolone, mirtazapine, and mefloquine. Interleukin-7 (IL-7) therapy reconstituted his lymphocytes and reduced his CSF JCV load. One month after IL-7 therapy, he developed worsening ataxia and CSF inflammation, which raised suspicion for immune reconstitution inflammatory syndrome. Steroids were restarted and his ataxia stabilized.
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